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Operative Techniques for Repair of Muscular Ventricular Septal Defects

      A ventricular septal defect (VSD) is defined as “a hole between the ventricular chambers or their remnants.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      A muscular ventricular septal defect is defined as “a VSD that has exclusively muscular borders.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      This article reviews the spectrum of these muscular ventricular septal defects, the challenges they can pose, and the surgical techniques that have a demonstrated track record of efficacy, with special consideration for the following subtypes of muscular ventricular septal defects: (1) midmuscular VSD or midventricular VSD, (2) inlet muscular VSD, (3) apical muscular VSD, (4) anterior trabecular VSD or infundibular muscular VSD, and (5) multiple VSD and “Swiss-cheese” VSD.

      Background

      A “VSD” is a “ventricular septal defect” and is also known as an “interventricular communication.” A VSD is defined as “a hole between the ventricular chambers or their remnants.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      The VSD is defined on the basis of its margins as seen from the aspect of the morphologically right ventricle. In the setting of double outlet right ventricle, the defect provides the outflow from the morphologically left ventricle. In univentricular atrioventricular connections with functionally single left ventricle with an outflow chamber, the communication is referred to by some as a bulboventricular foramen.
      A muscular ventricular defect is defined as “a VSD that has exclusively muscular borders.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      In an era when enthusiasm for transcatheter device closure of muscular VSDs is increasingly gaining attention and popularity, it is appropriate and important to review the spectrum of these defects, the challenges they can pose, and the surgical techniques that have a demonstrated track record of efficacy. This article considers these topics for the entire spectrum of muscular ventricular septal defects, a spectrum that includes the following subtypes (Fig. 1)
      • Jacobs J.P.
      • Burke R.P.
      • Quintessenza J.A.
      • et al.
      Congenital Heart Surgery Nomenclature and Database Project: Ventricular septal defect The Annals of Thoracic SurgeryApril 2000 Supplement.
      :
      • 1
        Midmuscular VSD or midventricular VSD
      • 2
        Inlet muscular VSD
      • 3
        Apical muscular VSD
      • 4
        Anterior trabecular VSD or infundibular muscular VSD
      • 5
        Multiple VSD and “Swiss-cheese” VSD.
      This article does not discuss indications or surgical techniques for banding of the pulmonary artery, a palliative strategy that may be quite useful in specific situations including some neonates or infants with apical VSDs or multiple VSDs, as well as small patients with familial objections to blood transfusions, and patients with complex associated defects.
      Figure thumbnail gr1
      Figure 1This diagram depicts the location in the ventricular septum of the following types of VDSs: perimembranous (inlet, trabecular, and outlet), doubly committed and juxta-arterial, and muscular (midmuscular VSD or midventricular VSD, inlet muscular VSD, apical muscular VSD, and anterior trabecular VSD or infundibular muscular VSD). Ant. = anterior; VSD = ventricular septal defect.

      Midmuscular VSD or Midventricular VSD

      A midmuscular VSD or midventricular VSD is defined as “a muscular VSD located in the central muscular septum.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      This type of VSD is perhaps the most “straightforward” of the muscular ventricular septal defects; however, closure of this type of VSD can be challenging because of the possibility of multiple right-sided openings of a defect that is a single opening on the left ventricular side. Difficulty in identifying the true margins of the VSD among the multiple trabeculations of the right side of the ventricular septum represents the main difficulty in closing this type of VSD. It is often necessary to divide some of the coarse muscular trabeculations on the right ventricular surface of the septum to expose the true margins of the VSD.
      • de Leval M.
      Ventricular septal defects.
      • Serraf A.
      • Lacour-Gayet F.
      • Bruniaux J.
      • et al.
      Surgical management of isolated multiple ventricular septal defects Logical approach in 130 cases.
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      The coarse multiple trabeculations on the right ventricular side of the septum, including the moderator band and lower end of the septal band, can be divided to facilitate accurate exposure of midmuscular VSDs as necessary. This technique can facilitate successful closure of defects that may appear to be multiple but are in fact single defects with more than one opening on the right ventricular side.
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      Common options for closure of midmuscular VSDs include surgical closure via a right atriotomy, surgical closure via a right ventriculotomy, and transcatheter device closure. These defects can usually be approached surgically through the right atrium using a standard right atriotomy (Fig. 2A-C). Large defects are closed with a patch sutured to the margins of the VSD on the right ventricular side of the septum. An oversized patch may be used to close multiple VSDs. Kitagawa and colleagues have described “placing the patch on the left ventricular side of the VSD for more secure closure … . The sutures are brought back across the septum from the left to the right side and secured over felt buttresses away from the edges of the defect. The patch is held in position on the left side of the septum by the sutures and the higher left ventricular pressures” (Fig. 2B and C insets).
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      Smaller defects may be closed with pledgeted horizontal mattress sutures.
      • de Leval M.
      Ventricular septal defects.
      The atrioventricular node and bundle of His, as well as the main bundle branches of the conduction tissue, are all remote from the margins of this type of VSD. Only terminal portions of the bundle branches may be near this VSD.
      • de Leval M.
      Ventricular septal defects.
      Figure thumbnail gr2
      Figure 2This diagram depicts closure of a midmuscular VSD and an anterior muscular VSD through a right atriotomy. (A) The view through the tricuspid valve of closely related midmuscular and anterior muscular VSDs. One may delineate the margins of the defects and improve exposure by dividing coarse right ventricular muscular trabeculations, including the septal and moderator bands. (B) The placement of an oversized patch to close these two closely related midmuscular and anterior muscular VSDs. The patch is cut slightly larger than the total area of VSDs. Multiple mattress sutures are placed through the patch, and then the patch and sutures are placed across the ventricular septum and into the left ventricle.
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      (B, inset) The patch on the left ventricular side of the ventricular septum. (C) The sutures have been brought back through the ventricular septum from the left to the right side and then passed through pledgets of felt and tied. (C, inset) The patch is held in position on the left side of the ventricular septum by both the sutures and the higher pressures in the left ventricle.
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      Ant. = anterior; LV = left ventricle; RV = right ventricle; VSD = ventricular septal defect.
      At times, closure through a right atriotomy is difficult and a right ventriculotomy may be necessary. When a right ventriculotomy is performed, the VSD is first assessed through a right atriotomy. A vertical right ventriculotomy is then made at least 5 mm away from the left anterior descending coronary artery, to facilitate improved exposure of the VSD. Standard patch closure may be performed via the right ventriculotomy. Alternatively, an external buttress technique may be utilized through a right ventriculotomy. This technique uses two pledget strips, one inside the right ventricle and one outside the right ventricle, which are tightly approximated with multiple horizontal mattress sutures that are placed from below the defect through the anterior right ventricular free wall and tied over the pledget strip outside the heart (Fig. 3).
      • de Leval M.
      Ventricular septal defects.
      Figure thumbnail gr3
      Figure 3This diagram demonstrates an external buttress technique that may be utilized to close muscular VSDs through a right ventriculotomy. This technique uses two pledget strips of felt, one inside the right ventricle and one outside the right ventricle, which are tightly approximated with multiple horizontal mattress sutures that are placed from below the defect through the anterior right ventricular free wall and tied over the pledget strip outside the heart.
      • de Leval M.
      Ventricular septal defects.
      Several additional approaches to closing midmuscular VSDs have been described. A “sandwich technique” using a patch with one side on the right ventricular surface and one side on the left ventricular surface has also been described (Fig. 4A-F).
      • Yamaguchi M.
      • Yoshimura N.
      • Oka S.
      • et al.
      Closure of muscular VSD by a sandwiching method via a coexisting larger VSD or an interatrial septostomy.
      • Ootaki Y.
      • Yamaguchi M.
      • Yoshimura N.
      • et al.
      Surgical management of trabecular ventricular septal defects The sandwich technique.
      • Murakami H.
      • Yoshimura N.
      • Takahashi H.
      • et al.
      Closure of multiple ventricular septal defects by the felt sandwich technique: Further analysis of 36 patients.
      • Brizard C.P.
      • Olsson C.
      • Wilkinson J.L.
      New approach to multiple ventricular septal defect closure with intraoperative echocardiography and double patches sandwiching the septum.
      Bacha and colleagues have described perventricular device closure of muscular ventricular septal defects on the beating heart.
      • Bacha E.A.
      • Cao Q.-L.
      • Starr J.P.
      • et al.
      Perventricular device closure of muscular ventricular septal defects on the beating heart: Technique and results.
      This technique of perventricular device closure technique can be utilized for midmuscular VSDs and also muscular VSDs in other locations in the ventricular septum; we illustrate this technique in the section about apical VSDs. Finally, nonsurgical transcatheter device closure of muscular ventricular septal defects has been described (Fig. 5).
      Figure thumbnail gr4
      Figure 4(A-F) This diagram demonstrates the “sandwich technique” using a patch with one side on the right ventricular surface and one side on the left ventricular surface.
      • Yamaguchi M.
      • Yoshimura N.
      • Oka S.
      • et al.
      Closure of muscular VSD by a sandwiching method via a coexisting larger VSD or an interatrial septostomy.
      • Ootaki Y.
      • Yamaguchi M.
      • Yoshimura N.
      • et al.
      Surgical management of trabecular ventricular septal defects The sandwich technique.
      • Murakami H.
      • Yoshimura N.
      • Takahashi H.
      • et al.
      Closure of multiple ventricular septal defects by the felt sandwich technique: Further analysis of 36 patients.
      • Brizard C.P.
      • Olsson C.
      • Wilkinson J.L.
      New approach to multiple ventricular septal defect closure with intraoperative echocardiography and double patches sandwiching the septum.
      (A) Two right angle clamps. One right angle clamp is passed through the right atrium, tricuspid valve, right ventricle, and VSD. A second right angle clamp is passed through the right atrium, patent foramen ovale, left atrium, mitral valve, left ventricle, and potentially the VSD. (B) The right angled clamps are used to pass a loop of suture across the VSD. (C) The loop of suture in position taking the following pathway: through the right atrium, tricuspid valve, right ventricle, VSD, left ventricle, mitral valve, left atrium, patent foramen ovale, and right atrium. (D) The loop of suture was passed through a patch and the patch was pulled through the right atrium, patent foramen ovale, left atrium, mitral valve, and left ventricle to sit on the left ventricular side of the ventricular septum. (E) The suture loop has now been passed through a second patch that is slid down the loop through the right atrium, tricuspid valve, and right ventricle to sit on the right ventricular side of the ventricular septum. (F) The “sandwich patch” in final position. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle; VSD = ventricular septal defect.
      Figure thumbnail gr5
      Figure 5This diagram demonstrates nonsurgical transcatheter device closure of a muscular ventricular septal defect.
      • Holzer R.
      • Balzer D.
      • Cao Q.-L.
      • et al.
      Amplatzer Muscular Ventricular Septal Defect Investigators
      Device closure of muscular ventricular septal defects using the Amplatzer muscular ventricular septal defect occluder: Immediate and mid-term results of a U.S. registry.
      (A) A midmuscular VSD. (B) A catheter passed through the superior vena cava, right atrium, tricuspid valve, right ventricle, VSD, and left ventricle. (C) Release of the left ventricular side of the device. (D) Release of the right ventricular side of the device. (E) The device is completely deployed. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.

      Inlet Muscular VSD

      An inlet muscular VSD is defined as “a muscular VSD located beneath the septal leaflet of the tricuspid valve.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      An inlet muscular VSD is typically closed through a right atriotomy. The surgical technique is similar to that previously described for a midmuscular VSD (Fig. 2A-C). However, special care must be taken to avoid damage to the conduction tissue (Fig. 6).
      • de Leval M.
      Ventricular septal defects.
      In contradistinction to the more common perimembranous inlet VSD, the muscular inlet VSD is surrounded by a complete rim of muscular tissue. The atrioventricular conduction axis penetrates into the ventricles on the superior (or leftward) side of the inlet muscular VSD. This location of the conduction tissue in relation to the inlet muscular VSD is completely different from the location of the conduction tissue in a perimembranous VSD or an atrioventricular canal-type defect. In the muscular inlet VSD, the conduction tissue is close to the outlet aspect of the VSD, in other words, on the surgeon's left hand side when viewing the defect through the tricuspid valve in a heart in situs solitus with concordant atrioventricular connections. In this location, sutures must be placed superficially on the right ventricular side of the ventricular septum near the superior rim of the VSD. Because of the proximity to the atrioventricular valves and conduction tissue, this type of VSD is a poor candidate for closure with a device, and in some cases may not be amenable to closure with a device.
      Figure thumbnail gr6
      Figure 6This diagram demonstrates a heart with both a perimembranous inlet VSD and a muscular inlet VSD. The exposure is through the right atrium and tricuspid valve. The conduction tissue is shown in the bridge of muscle between the two VSDs. The conduction tissue is related to perimembranous inlet VSD and the muscular inlet VSD in the usual position relative to each VSD. Because the bridge of muscle between the two VSDs contains the conduction tissue and may be quite narrow, these two VSDs should be closed with a single patch to avoid heart block.
      • de Leval M.
      Ventricular septal defects.
      • Bharati S.
      • Lev M.
      • Kirklin J.W.
      Cardiac Surgery and the Conduction System.
      Figure 6 demonstrates a heart with both a perimembranous VSD and a muscular inlet VSD approached through a right atriotomy and the tricuspid valve. The conduction tissue is in the bridge of muscle between the two VSDs. It is related to the perimembranous VSD and the muscular VSD in the usual position relative to each VSD. Because the bridge of muscle between the two VSDs contains the conduction tissue and may be quite narrow, these two VSDs should be closed with a single patch to avoid heart block.
      • de Leval M.
      Ventricular septal defects.
      • Bharati S.
      • Lev M.
      • Kirklin J.W.
      Cardiac Surgery and the Conduction System.
      This situation wherein an inlet muscular VSD and a perimembranous VSD coexist is not analogous to the heart with multiple muscular defects. In the heart where an inlet muscular VSD and a perimembranous VSD coexist, closure with a single patch over both holes is recommended to avoid injury to the conduction system. In this heart, division or excision of the strip of muscle between the inlet muscular VSD and a perimembranous VSD is certain to destroy the conduction system and create permanent third-degree heart block.

      Apical Muscular VSD

      An apical muscular VSD is defined as “a muscular VSD located apically relative to the moderator band.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      Apical muscular VSDs can be technically challenging to close. Closure through a right atriotomy can be especially difficult due to limited exposure. Closure options include surgical approaches through a right ventriculotomy or left ventriculotomy, hybrid approaches with perventricular device closure, and transcatheter device closure.
      Stellin and colleagues described surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy (Fig. 7).
      • Stellin G.
      • Padalino M.
      • Milanesi O.
      • et al.
      Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy.
      Tsang and colleagues described an alternative approach to close apical muscular VSDs through a through a modified apical right ventriculotomy (Fig. 8).
      • Tsang V.T.
      • Hsia T.-Y.
      • Yates R.W.M.
      • et al.
      Surgical repair of supposedly multiple defects within the apical part of the muscular ventricular septum.
      Another alternative approach is through an apical left ventriculotomy. Although some authors have reported diminished left ventricular function or late aneurysm formation with this approach, the left ventricular apical approach can be very useful.
      • de Leval M.
      Ventricular septal defects.
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      • Singh A.K.
      • de Leval M.R.
      • Stark J.
      Left ventriculotomy for closure of muscular ventricular septal defects Treatment of choice.
      • McDaniel N.
      • Gutgesell H.P.
      • Nolan S.P.
      • et al.
      Repair of large muscular ventricular septal defects in infants employing left ventriculotomy.
      • Hannan R.
      Repair of large muscular ventricular septal defects in infants employing left ventriculotomy.
      The ventriculotomy incision should be kept as small as possible and limited to the true apex of the left ventricle (Fig. 9). Bacha and colleagues have described perventricular device closure of apical muscular VSDs on the beating heart (Fig. 10).
      • Bacha E.A.
      • Cao Q.-L.
      • Starr J.P.
      • et al.
      Perventricular device closure of muscular ventricular septal defects on the beating heart: Technique and results.
      Figure thumbnail gr7
      Figure 7This figure shows closure of an apical muscular VSD through a right ventricular apical infundibulotomy as described by Stellin and colleagues.
      • Stellin G.
      • Padalino M.
      • Milanesi O.
      • et al.
      Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy.
      (A) An apical infundibulotomy that is made parallel to the distal portion of left anterior descending (LAD) coronary artery. (B) The exposed apical ventricular septal defect. In this diagram, and in some cases of apical ventricular septal defects, the defect extends above and below the moderator band.
      • Stellin G.
      • Padalino M.
      • Milanesi O.
      • et al.
      Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy.
      LAD = left anterior descending coronary artery; LV = left ventricle; RV = right ventricle.
      Figure thumbnail gr8
      Figure 8This figure shows closure of an apical muscular VSD through a modified apical right ventriculotomy as described by Tsang and colleagues.
      • Tsang V.T.
      • Hsia T.-Y.
      • Yates R.W.M.
      • et al.
      Surgical repair of supposedly multiple defects within the apical part of the muscular ventricular septum.
      The patch to close the VSD is anchored to the left ventricular surface posteriorly, and within the right ventricular surface anteriorly. (A) The anatomy in short axis. (B) The anatomy in the four-chamber projection. Ant. = anterior; LV = left ventricle; RV = right ventricle; VSD = ventricular septal defect.
      Figure thumbnail gr9
      Figure 9This figure shows closure of an apical muscular VSD through a left ventricular apical approach.
      • de Leval M.
      Ventricular septal defects.
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      • Singh A.K.
      • de Leval M.R.
      • Stark J.
      Left ventriculotomy for closure of muscular ventricular septal defects Treatment of choice.
      • McDaniel N.
      • Gutgesell H.P.
      • Nolan S.P.
      • et al.
      Repair of large muscular ventricular septal defects in infants employing left ventriculotomy.
      • Hannan R.
      Repair of large muscular ventricular septal defects in infants employing left ventriculotomy.
      A small “fish-mouth”-type incision can be made at the apex of the left ventricle parallel to and away from the LAD. This allows exposure of the VSD. LAD = left anterior descending coronary artery; LV = left ventricle; RV = right ventricle.
      Figure thumbnail gr10
      Figure 10(A-D) This figure shows closure of multiple muscular VSDs with perventricular placement of a device on a beating heart as described by Bacha and colleagues.
      • Bacha E.A.
      • Cao Q.-L.
      • Starr J.P.
      • et al.
      Perventricular device closure of muscular ventricular septal defects on the beating heart: Technique and results.
      (A) Multiple muscular VSDs with a wire across the largest VSD. In (B), the wire is positioned across the largest defect, and the sheath is positioned with its tip in the left ventricular cavity. The device is then pushed in and deployed by slowly pulling the sheath back. (C) Release of the left ventricular side of the device. (D) The device is completely deployed. VSD = ventricular septal defect.

      Anterior Trabecular VSD or Infundibular Muscular VSD

      An outlet muscular VSD is defined as “a muscular VSD that opens between the septal band (septomarginal trabeculation) and the semilunar valve.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      These defects include VSDs known as “anterior trabecular VSDs” and “infundibular muscular VSDs.” This type of VSD is encountered in approximately 15 to 20% of patients with tetralogy of Fallot (TOF),
      • de Leval M.
      Ventricular septal defects.
      and may be a secondary VSD in TOF or the only VSD in TOF.
      Because of the proximity to the semilunar valves, this type of VSD is a poor candidate for closure with a device, and in some cases may not be amenable to closure with a device. The infundibular muscular VSD is separated from the conduction tissue inferiorly and posteriorly by the fusion of the posterior limb of the trabecula septomarginalis with the ventriculo-infundibular fold (Fig. 11).
      • de Leval M.
      Ventricular septal defects.
      Figure thumbnail gr11
      Figure 11This figure shows an outlet muscular VSD, which is defined as “a muscular VSD that opens between the septal band (septomarginal trabeculation) and the semilunar valve.”
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      These defects include VSDs, known as “anterior trabecular VSDs” and “infundibular muscular VSDs.” This type of VSD is in close proximity to the ventriculo-arterial valves. The infundibular muscular VSD is separated from the conduction tissue inferiorly and posteriorly by the fusion of the posterior limb of the trabecula septomarginalis with the ventriculo-infundibular fold.
      • de Leval M.
      Ventricular septal defects.
      PA = pulmonary artery; TV = tricuspid valve.
      Muscular outlet VSDs may be approached through the right atrium, right ventricle, or pulmonary artery. Most commonly, they are approached through a right ventriculotomy with division of muscle bundles to expose the VSD. Anterior defect exposure is sometimes facilitated by a transverse pulmonary arteriotomy.
      • Alsoufi B.
      • Karamlou T.
      • Osaki M.
      • et al.
      Surgical repair of multiple muscular ventricular septal defects: The role of re-endocardialization strategy.
      These VSDs are surrounded entirely by muscle and closed with a patch sewn to the margins of the defect.

      Multiple VSD and “Swiss-Cheese” VSD

      Multiple muscular VSDs represent a surgical challenge. The term “VSD, Type 4 (Muscular), Multiple-“Swiss-Cheese”” is defined by the Congenital Heart Surgery Database of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons (STS) as a heart where “more than three muscular VSDs exist.”
      • Jacobs J.P.
      • Burke R.P.
      • Quintessenza J.A.
      • et al.
      Congenital Heart Surgery Nomenclature and Database Project: Ventricular septal defect The Annals of Thoracic SurgeryApril 2000 Supplement.
      A variety of definitions have been used to define this entity,
      • Kitagawa T.
      • Durham III, L.A.
      • Mosca R.S.
      • et al.
      Technique and results in the management of multiple ventricular septal defects.
      • Fishberger S.B.
      • Bridges N.D.
      • Keane J.F.
      • et al.
      Intraoperative device closure of ventricular septal defects.
      • Leca F.
      • Karam J.
      • Vouhè P.R.
      • et al.
      Surgical treatment of multiple ventricular septal defects using a biologic glue.
      including uncountable multiple VSDs.
      • Leca F.
      • Karam J.
      • Vouhè P.R.
      • et al.
      Surgical treatment of multiple ventricular septal defects using a biologic glue.
      Nevertheless, for the purpose of these databases, this term is utilized if four or more muscular VSDs exist, as defined by Serraf and colleagues.
      • Serraf A.
      • Lacour-Gayet F.
      • Bruniaux J.
      • et al.
      Surgical management of isolated multiple ventricular septal defects Logical approach in 130 cases.
      It is desirable to specify the number of orifices from both the left ventricular and the right ventricular aspects, because clearly, these may be different.
      The International Working Group for Defining the Nomenclatures for Paediatric and Congenital Heart Disease (Definitions Working Group) of The International Society for Nomenclature of Paediatric and Congenital Heart Disease.
      Challenges with multiple VSDs and a “Swiss-cheese” septum include the risk of conduction system injury in the area between VSDs, and the difficulty in identifying and closing all of the holes. A combination of any of the strategies previously described may be useful for the closure of multiple VSDs and a “Swiss-cheese” septum. Occasionally, a larger oversized patch may be used to cover multiple holes. Occasionally, multiple cardiac incisions may be necessary in the same operation, including combined right atriotomy, right ventriculotomy, and left ventriculotomy.
      A preoperatively placed cardiac catheter can allow accurate localization of muscular VSDs and especially apical muscular VSDs. The approach can improve visualization of the VSD from the right side of the heart and also minimize the size of the ventriculotomy required for satisfactory closure (Fig. 12).
      • Thomson J.D.R.
      • Gibbs J.L.
      • van Doorn C.
      Cardiac catheter guided surgical closure of an apical ventricular septal defect.
      This strategy, as well as the sandwich technique previously described, may both be used to facilitate hybrid intraoperative device deployment.
      Figure thumbnail gr12
      Figure 12A preoperatively placed cardiac catheter can allow accurate localization of muscular VSDs and especially apical muscular VSDs. The approach can improve visualization of the VSD from the right side of the heart and also minimize the size of the ventriculotomy required for satisfactory closure. This drawing shows the catheter, which had been placed retrograde from the aorta through the apical ventricular septal defect into the right side of the heart. The arrows represent the direction of passage of the catheter.
      • Thomson J.D.R.
      • Gibbs J.L.
      • van Doorn C.
      Cardiac catheter guided surgical closure of an apical ventricular septal defect.
      LA = left atrium; LV = left ventricle; MV = mitral valve; RA = right atrium; RV = right ventricle; TV = tricuspid valve; VSD = ventricular septal defect.
      Multiple apical VSDs can be treated by apical exclusion using the “septal obliteration technique,” where a patch is sewn in position to exclude a portion of the right ventricular apex from the remainder of the right ventricular cavity. This technique creates a “noncommunicating” left-to-right ventricular shunt at the “blind-ending” neoapical chamber that is separated from the remainder of the right ventricle by the patch of the “septal obliteration technique.”
      • Black M.D.
      • Shukla V.
      • Rao V.
      • et al.
      Repair of isolated multiple muscular ventricular septal defects: The septal obliteration technique.
      Finally, the team from Toronto has described a technique named “transatrial re-endocardialization” to close multiple muscular ventricular septal defects. Exposure is through a right atriotomy, although anterior defect exposure was sometimes facilitated by a transverse pulmonary arteriotomy. A ventriculotomy was not used to access the VSDs unless it was required for repair of associated defects. Identification of muscular VSDs was done by gentle probing of trabeculations with a right angle clamp to delineate a connection with the left ventricle. Probing was also done through a perimembranous VSD or through the interatrial septum, to help completely identify all muscular VSDs. Large VSDs were closed with patches. Multiple smaller VSDs were closed with transatrial re-endocardialization, using “6-0 or 7-0 polypropylene sutures with a BV1 or BV175 needle (Ethicon, Inc, Somerville, NJ) (Fig. 13). The septal trabeculations were approximated in two layers of superficial, endocardial running sutures. Each suture was started at the distal edge of a trabeculation and run in a double-layer, simple, continuous fashion to the other end of the trabeculation, where they were tied … . It was sometimes possible to close multiple trabeculations with the same suture.”
      • Alsoufi B.
      • Karamlou T.
      • Osaki M.
      • et al.
      Surgical repair of multiple muscular ventricular septal defects: The role of re-endocardialization strategy.
      Figure thumbnail gr13
      Figure 13This drawing shows the technique described by the team from Toronto named “transatrial re-endocardialization” to close multiple muscular ventricular septal defects. (A) The preoperative appearance of a heart with multiple ventricular septal defects, including multiple muscular defects involving the midmuscular, apical, and anterior compartments. (B) The re-endocardialization strategy used on smaller VSDs that uses a “double-layer suturing of septal trabeculations to each other with fine, superficial, endocardial running sutures.” (C) The patch closure of a larger midmuscular VSD. (D) Complete obliteration of ventricular septum with patch repair of the large midmuscular VSD and re-endocardialization sutures to obliterate completely all of the remaining VSDs. VSD = ventricular septal defect.

      Discussion

      In the STS Congenital Heart Surgery Database, during the 4-year time period of 2005 through 2008 inclusive, 75,087 operations were analyzed.
      • Jacobs J.P.
      • Jacobs M.L.
      • Mavroudis C.
      • et al.
      Executive summary: The Society of Thoracic Surgeons Congenital Heart Surgery Database—Tenth Harvest (January 1, 2005-December 31, 2008) The Society of Thoracic Surgeons (STS) and Duke Clinical Research Institute (DCRI), Duke University Medical Center, Durham, North Carolina, United States, Spring 2009 Harvest.
      Of these 75,087 operations, 4752 were classified as having VSD repair as their primary procedure. Table 1 shows the incidence of the various subtypes of VSD that compose these 4752 operations.
      Table 1Incidence of the Various Subtypes of VSD
      VSD TypeNumberPercent of All VSD
      VSD, Single48210.1
      VSD, Type 1 (Subarterial) (Supracristal) (Conal septal defect) (Infundibular)3587.5
      VSD, Type 2 (Perimembranous) (Paramembranous) (Conoventricular)333070.1
      VSD, Type 3 (Inlet) (AV canal type)1423.0
      VSD, Type 4 (Muscular)1853.9
      VSD, Type: Gerbode type (LV-RA communication)160.3
      VSD, Multiple2395.0
      All VSD4752100.0
      AV, atrioventricular; LV, left ventricle; RA, right atrium; VSD, ventricular septal defect.
      Table 2 shows cardiopulmonary bypass time in minutes, as well as the rates of heart block, unplanned reoperation prior to hospital discharge, and discharge mortality, for the various subtypes of VSD.
      Table 2Bypass Time and Outcome Data for the Various Subtypes of VSD
      VSD TypeCPB Time (min)Heart BlockUnplanned ReoperationDischarge Mortality
      VSD, Single72.81.7%2.5%0.8%
      VSD, Type 1 (Subarterial) (Supracristal) (Conal septal defect) (Infundibular)72.70.6%2.2%0.6%
      VSD, Type 2 (Perimembranous) (Paramembranous) (Conoventricular)750.9%1.3%0.5%
      VSD, Type 3 (Inlet) (AV canal type)90.83.5%2.8%1.4%
      VSD, Type 4 (Muscular)79.92.7%4.3%1.1%
      VSD, Type: Gerbode type (LV-RA communication)67.90.0%0.0%0.0%
      VSD, Multiple111.22.9%2.5%1.7%
      AV, atrioventricular; CPB, cardiopulmonary bypass; LV, left ventricle; RA, right atrium; VSD, ventricular septal defect.
      These data from the STS Congenital Heart Surgery Database demonstrate the following concepts:
      • 1
        Multiple VSDs take longer to close.
      • 2
        When compared with the more common perimembranous VSD, the rate of heart block is three times higher with muscular VSDs and multiple VSDs.
      • 3
        When compared with the more common perimembranous VSD, the rate of unplanned reoperation is over four times higher with muscular VSDs and almost twice as high with multiple VSDs.
      • 4
        When compared with the more common perimembranous VSD, the rate of mortality before discharge from the hospital is over twice as high with muscular VSDs and over three times as high with multiple VSDs.
      In summary, the surgical management of muscular VSDs and multiple VSDs is a challenging area. These operations are associated with increased mortality and morbidity in comparison to closure of the more common perimembranous VSD. Congenital heart surgeons must be aware of a variety of techniques that are available to close muscular VSDs. The choice of the appropriate technique(s) is critical to the success of the surgical exercise.

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